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Dermatology Online Journal

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Hailey-Hailey disease exacerbated by multiple pregnancies: case report and review of the literature


Hailey-Hailey disease is a rare chronic skin disorder that is inherited in an autosomal dominant manner. The disease is characterized by development of vesicles and bullae typically in the intertriginous areas. On histology, there is widespread intraepidermal acantholysis causing the “dilapidated brick-wall” appearance. Mutations in the ATP2C1 gene, encoding for P-type Ca2+ transport ATPase, is the primary cause of the disease. The disease manifests around puberty and runs a chronic course with remissions and exacerbations. Ultraviolet light exposure, sweating, friction, stress, and cutaneous infections are the known precipitants of the disease. We report a case of a woman with recurrent flare-ups of Hailey-Hailey disease with repeated pregnancies and remission of her disease during non-pregnancy periods. To our knowledge, this is the first reported case of Hailey-Hailey disease exacerbated by pregnancy.

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